Hypertrophic cardiomyopathy: A heart in need of an energy bar?

Styliani Vakrou, M. Roselle Abraham

Research output: Contribution to journalReview article

Abstract

Hypertrophic cardiomyopathy (HCM) has been recently recognized as the most common inherited cardiovascular disorder, affecting 1 in 500 adults worldwide. HCM is characterized by myocyte hypertrophy resulting in thickening of the ventricular wall, myocyte disarray, interstitial and/or replacement fibrosis, decreased ventricular cavity volume and diastolic dysfunction. HCM is also the most common cause of sudden death in the young. A large proportion of patients diagnosed with HCM have mutations in sarcomeric proteins. However, it is unclear how these mutations lead to the cardiac phenotype, which is variable even in patients carrying the same causal mutation. Abnormalities in calcium cycling, oxidative stress, mitochondrial dysfunction and energetic deficiency have been described constituting the basis of therapies in experimental models of HCM and HCM patients. This review focuses on evidence supporting the role of cellular metabolism and mitochondria in HCM.

Original languageEnglish (US)
Article numberArticle 309
JournalFrontiers in Physiology
Volume5 AUG
DOIs
StatePublished - 2014

Keywords

  • Bioenergetic deficit
  • Calcium handling
  • Hypertrophic cardiomyopathy
  • Induced pluripotent stem cells (iPSCs)
  • Mitochondria

ASJC Scopus subject areas

  • Physiology
  • Physiology (medical)

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